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Force, falls and fear of falls in myotonic dystrophy type 1

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Force, falls and fear of falls in myotonic

dystrophy type 1

Cross-sectional and longitudinal studies

Akademisk avhandling

som för avläggande av medicine doktorsexamen

vid Sahlgrenska akademin vid Göteborgs Universitet kommer att offentligen försvaras i

hörsal Arvid Carlsson, Academicum, Medicinargatan 3, Göteborg,

Fredagen den 17 oktober 2014, kl 9:00

av

Elisabet Hammarén

Legitimerad sjukgymnast

Fakultetsopponent:

Docent Britta Lindström

Enheten för fysioterapi

Institutionen för samhällsmedicin och rehabilitering

Umeå universitet

Avhandlingen baseras på följande delarbeten:

I. Hammarén E, Ohlsson JA, Lindberg C, Kjellby-Wendt G. Reliability of

static and dynamic balance tests in subjects with myotonic dystrophy type 1.

Adv Physiother 2012; 14: 48–54. With Appendix.

II. Hammarén E, Kjellby-Wendt G, Kowalski J, Lindberg C. Factors of

importance for dynamic balance impairment and frequency of falls in

individuals with myotonic dystrophy type 1 – A cross-sectional study –

Including reference values of Timed Up & Go, 10 m walk and step test.

Neuromuscul Disord 2014;24(3)207-15.

III. Hammarén E, Kjellby-Wendt G, Lindberg C. Muscle force, balance and

falls in muscular impaired individuals with myotonic dystrophy type 1 - A

five-year prospective cohort study.

Submitted

2014

(2)

Force, falls and fear of falls in myotonic dystrophy type 1

Cross-sectional and longitudinal studies

Elisabet Hammarén

Department of Clinical Neuroscience and Rehabilitation, Institute of Neuroscience and Physiology Sahlgrenska Academy at University of Gothenburg

ABSTRACT

Background: Myotonic dystrophy type 1 (DM1) is a neuromuscular multi-systemic disorder with slowly progressive muscle weakness. The overall purpose of this thesis was, in adult patients with DM1, to investigate factors of importance for functional balance skills and falls, and to investigate the natural course of muscle force and functional balance impairments, with reliable measurement methods.

Methods: In the first study we evaluated test-retest reliability in static and dynamic balance tests and gait, with three assessment occasions spaced one-week apart, in ten patients with DM1. In the second study, which is a cross-sectional study, 51 patients were assessed for muscle strength, gait and functional balance together with self-reported balance confidence, walking ability and falls. A multivariate analysis of factors of importance for functional balance impairment was performed. Of these 51 patients, 43 were further analysed in a third five-year prospective study for changes in muscle force, gait and functional balance together with self-reported balance confidence, walking ability and falls.

Results: The test-retest reliability analysis results advocate dynamic balance tests and timed gait before the static tests. The cross-sectional study shows that falls are common in the weaker, but still ambulant, patients. A combination of weak ankle muscles and a physical capability to accelerate to fast walking increased the risk of falling. Over five years the distal muscles of the leg have a more steep force decrease than the proximal muscles. There was a tendency towards a greater worsening in males, and we found a statistically significant difference between genders in the knee extensor and flexor force change. All men had fallen within the previous year at the five-year assessment. Injuries of the face and head were more frequent at five years.

Conclusions: Test-retest reliable dynamic balance tests and isometric muscle force measures showed that there is a statistically significant decrease in functional balance skill and in leg muscle force after five years in patients with DM1. The number of patients who had fallen had increased and the fall injuries were worse. It is of great importance to prevent falls especially in those who are at most risk for falls, by which we mean those who have a more steep muscle force reduction. Regular assessments of gait, functional balance and leg muscle force could be a way to determine who is at most risk for falls. This would give the opportunity to intervene with rehabilitation therapy and assistive devices as possible means for fall prevention in patients with DM1.

Keywords: myotonic dystrophy, physiotherapy, muscle force, postural balance, gait, reliability, cross- sectional, prospective

ISBN: 978-91-628-9077-3 (tryckt) ISBN: 978-91-628-9098-8 (e-pub)

http://hdl.handle.net/2077/35958

References

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